Article
Oncology
Katsuyuki Miyabe, Vinay Chandrasekhara, Nicha Wongjarupong, Jun Chen, Lu Yang, Stephen Johnson, Nicholas Chia, Marina Walther-Antonio, Janet Z. Yao, Sean C. Harrington, Cynthia K. Nordyke, John E. Eaton, Andrea A. Gossard, Sharad Oli, Hamdi A. Ali, Sravanthi Lavu, Nasra H. Giama, Fatima A. Hassan, Hawa M. Ali, Felicity T. Enders, Sumera I. Ilyas, Gregory J. Gores, Mark D. Topazian, Purna C. Kashyap, Lewis R. Roberts
Summary: This study investigated the microbiota profiles in bile and stool samples of patients with PSC and CCA, and found that bile and stool have different microbiota profiles. The abundance of Fusobacteria in bile was correlated with the duration of PSC in CCA patients. These findings suggest a potential role for microbiota-driven inflammation in the pathogenesis of perihilar CCA.
Article
Gastroenterology & Hepatology
Maria Reich, Lina Spomer, Caroline Klindt, Katharina Fuchs, Jan Stindt, Kathleen Deutschmann, Johanna Hohne, Evaggelia Liaskou, Johannes R. Hov, Tom H. Karlsen, Ulrich Beuers, Joanne Verheij, Sofia Ferreira-Gonzalez, Gideon Hirschfield, Stuart J. Forbes, Christoph Schramm, Irene Esposito, Dirk Nierhoff, Peter Fickert, Claudia Daniela Fuchs, Michael Trauner, Maria Garcia-Beccaria, Gisela Gabernet, Sven Nahnsen, Jan-Philipp Mallm, Marina Vogel, Kristina Schoonjans, Tobias Lautwein, Karl Koehrer, Dieter Haeussinger, Tom Luedde, Mathias Heikenwalder, Verena Keitel
Summary: The study revealed that reduced levels of TGR5 in BECs from patients with PSC and Abcb4(-/-) mice promote the development of a reactive BEC phenotype, aggravate biliary injury, and contribute to the pathogenesis of sclerosing cholangitis. Restoration of biliary TGR5-expression levels represents a previously unknown mechanism of action of norUDCA.
JOURNAL OF HEPATOLOGY
(2021)
Article
Cell Biology
Christine L. Zimmer, Erik von Seth, Marcus Buggert, Otto Strauss, Laura Hertwig, Son Nguyen, Alicia Y. W. Wong, Chiara Zotter, Lena Berglin, Jakob Michaelsson, Marcus Reuterwall Hansson, Urban Arnelo, Ernesto Sparrelid, Ewa C. S. Ellis, Johan D. Soderholm, Asa Keita, Kristian Holm, Volkan Ozenci, Johannes R. Hov, Jeff E. Mold, Martin Cornillet, Andrea Ponzetta, Annika Bergquist, Niklas K. Bjorkstrom
Summary: The study on patients with primary sclerosing cholangitis (PSC) revealed the characteristics of the human biliary immunological landscape, highlighting differences in immune cell composition compared to blood and the pathological features of biliary inflammation in PSC patients.
SCIENCE TRANSLATIONAL MEDICINE
(2021)
Article
Surgery
Wisam Sbeit, Tawfik Khoury, Eran Goldin, Mahmud Mahamid
Summary: The study found that fully-covered metal stent (FCMS) was a safe and effective treatment option for patients with Primary Sclerosing Cholangitis (PSC) related refractory Dominant Strictures (DS).
SURGICAL ENDOSCOPY AND OTHER INTERVENTIONAL TECHNIQUES
(2022)
Article
Gastroenterology & Hepatology
Shekhar Singh Jadaun, Rohit Mehtani, Ana Hasnain, Sushant Bhatia, Vikash Moond, Mukesh Kumar, Vikash Kuhad, Shweta Singh, Shaleen Agarwal, Subhash Gupta, Sanjiv Saigal
Summary: This retrospective study analyzed the outcomes of living donor liver transplant (LDLT) in primary sclerosing cholangitis (PSC) at a tertiary liver transplant center in north India. The study found that LDLT in PSC can achieve good long-term outcomes, but there is a risk of PSC recurrence in approximately one-fifth of patients.
HEPATOLOGY INTERNATIONAL
(2023)
Review
Cell Biology
Marisol I. Gonzalez, Danielle T. Vannan, Bertus Eksteen, Iran Flores-Sotelo, Jose Luis Reyes
Summary: Cholestasis, which is impaired bile flow from the liver into the intestine, can be caused by cholangitis and/or bile duct obstruction. Immune-mediated cholangitis in primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) is characterized by a strong immune response targeting the biliary epithelial cells (BECs). Mast cells (MCs) have been found to play a role in biliary inflammation and neoplasia, suggesting that they are relevant players in these diseases.
Article
Gastroenterology & Hepatology
Christina Villard, Ingalill Friis-Liby, Fredrik Rorsman, Karouk Said, Anna Warnqvist, Martin Cornillet, Stergios Kechagias, Nils Nyhlin, Marten Werner, Izabella Janczewska, Therese Hagstrom, Emma Nilsson, Annika Bergquist
Summary: This study prospectively evaluated the effectiveness of yearly MRI/MRCP surveillance in detecting cholangiocarcinoma (CCA) in patients with primary sclerosing cholangitis (PSC) and found it to be ineffective in detecting cancer early enough to support long-term survival. Personalized follow-up strategies and improved diagnostic methods for PSC-related CCA are needed.
JOURNAL OF HEPATOLOGY
(2023)
Review
Biochemistry & Molecular Biology
Ji-Won Park, Jung-Hee Kim, Sung-Eun Kim, Jang Han Jung, Myoung-Kuk Jang, Sang-Hoon Park, Myung-Seok Lee, Hyoung-Su Kim, Ki Tae Suk, Dong Joon Kim
Summary: Cholangiopathies are various biliary diseases that affect the biliary epithelium and can lead to cholestasis and liver cirrhosis. Primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) are the most important progressive cholangiopathies in adults, with research focusing on genetic risk, epigenetic changes, dysregulated mucosal immunity, and altered biliary epithelial cell function. Understanding the molecular pathogenesis of these diseases can help clinicians prevent and treat them more effectively.
Article
Medicine, Research & Experimental
Raanan Greenman, Michal Segal-Salto, Neta Barashi, Ophir Hay, Avi Katav, Omer Levi, Ilan Vaknin, Revital Aricha, Sarit Aharoni, Tom Snir, Inbal Mishalian, Devorah Olam, Johnny Amer, Ahmad Salhab, Rifaat Safadi, Yaakov Maor, Palak Trivedi, Christopher J. Weston, Francesca Saffioti, Andrew Hall, Massimo Pinzani, Douglas Thorburn, Amnon Peled, Adi Mor
Summary: CCL24 is involved in fibrosis and inflammation in liver and its inhibition can reduce liver injury. This study found that blocking CCL24 improves inflammation, fibrosis, and cholestasis in PSC, and reduces proliferation and senescence of cholangiocytes.
Article
Gastroenterology & Hepatology
Roopa H. Nanjundappa, Urs Christen, Channakeshava S. Umeshappa
Summary: Primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) are two major autoimmune liver diseases. PBC primarily affects intrahepatic small bile duct epithelial cells in women, while PSC mainly affects medium and big bile duct epithelial cells in men. Cholangiocarcinoma (CCA) is a malignancy arising from cholangiocytes and is more prevalent in PSC patients than in PBC patients. Understanding the immune surveillance mechanisms that differentiate between PBC and PSC patients is crucial for developing effective therapies and early diagnosis of CCA.
HEPATOLOGY COMMUNICATIONS
(2023)
Article
Gastroenterology & Hepatology
Roopa H. Nanjundappa, Urs Christen, Channakeshava S. Umeshappa
Summary: Primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) are two significant autoimmune liver diseases. PBC primarily affects small bile duct epithelial cells in women, while PSC predominantly affects medium and large intrahepatic and extrahepatic bile duct epithelial cells in men. Cholangiocarcinoma (CCA) is a malignancy arising from cholangiocytes and its incidence is increasing worldwide. Although PBC patients rarely develop CCA compared to PSC patients, understanding the differences in immune surveillance mechanisms is crucial for effective diagnosis and treatment.
HEPATOLOGY COMMUNICATIONS
(2023)
Article
Multidisciplinary Sciences
Motoko Sasaki, Yasunori Sato, Yasuni Nakanuma
Summary: Accumulating studies show that senescent biliary epithelial cells produce senescence-associated secretory phenotypes and play various roles in the pathogenesis of primary biliary cholangitis and other cholangiopathies. Upregulated expression of various genes related to immunity and inflammation, including IFIT3, in senescent BECs may be associated with the pathogenesis of PBC.
SCIENTIFIC REPORTS
(2021)
Article
Gastroenterology & Hepatology
Aristeidis Grigoriadis, Kristina Imeen Ringe, Johan Bengtsson, Erik Baubeta, Cecilia Forsman, Nafsika Korsavidou-Hult, Fredrik Rorsman, Emma Nilsson, Nikolaos Kartalis, Annika Bergquist
Summary: Magnetic resonance cholangiopancreatography (MRCP) plays an important role in the prognosis assessment of primary sclerosing cholangitis (PSC). A novel, simple, and reproducible risk-score (DiStrict score) based on MRCP findings was developed and showed a strong association with prognosis in individuals with PSC. This score can be easily used in clinical practice and has the potential to be useful in clinical trials and patient counseling and management.
Article
Gastroenterology & Hepatology
John E. Eaton, Christopher L. Welle, Zeinab Bakhshi, Shannon P. Sheedy, Ilkay S. Idilman, Gregory J. Gores, Charles B. Rosen, Julie K. Heimbach, Timucin Taner, Denise M. Harnois, Keith D. Lindor, Nicholas F. LaRusso, Andrea A. Gossard, Konstantinos N. Lazaridis, Sudhakar K. Venkatesh
Summary: This study aimed to evaluate the diagnostic performances and prognostic implications of ultrasound and MRI-based perihilar cholangiocarcinoma (CCA) detection among patients with primary sclerosing cholangitis (PSC). Results showed that MRI was superior to ultrasound for the detection of early-stage CCA in PSC patients, and identifying CCA before symptom onset with MRI was associated with improved outcomes.
Article
Immunology
Emanual Maverakis, Alexander A. Merleev, Dayoung Park, Muchena J. Kailemia, Gege Xu, L. Renee Ruhaak, Kyoungmi Kim, Qiuting Hong, Qiongyu Li, Patrick Leung, William Liakos, Yu-Jui Yvonne Wan, Christopher L. Bowlus, Alina I. Marusina, Nelvish N. Lal, Yixuan Xie, Guillaume Luxardi, Carlito B. Lebrilla
Summary: The study utilized MRM mass spectrometry to characterize glycan alterations associated with PBC and PSC, resulting in multi-analyte diagnostic models capable of accurately distinguishing the diseases. Additionally, the research investigated how environmental factors, such as bile acids, can influence glycosylation and potentially lead to the generation of altered selfantigens as immune targets.
CLINICAL IMMUNOLOGY
(2021)